sign in sign up
<<
Home , Atrial enlargement, Cardiomegaly, Right atrial enlargement

ORIGINAL ARTICLE

Clinical Manifestation and Survival of Patients with Mizuhiro Arima, TatsujiI diopathicKanoh, Shinya BilateralOkazaki, YoshitakaAtrialIwama,DilatationAkira Yamasaki and Sigeru Matsuda

Westudied the histories of eight patients who lacked clear evidence of cardiac abnormalities other than marked bilateral atrial dilatation and atrial , which have rarely been dis- cussed in the literature. From the time of their first visit to our hospital, the patients' chest radio- graphs and electrocardiograms showed markedly enlarged cardiac silhouettes and atrial fibrilla- tion, respectively. Each patient's echocardiogram showed a marked bilateral atrial dilatation with almost normal wall motion of both ventricles. In one patient, inflammatory change was demonstrated by and endomyocardial biopsy from the right ventricle. Seven of our eight cases were elderly women.Over a long period after the diagnosis of cardiome- galy or , or digitalis offered good results in the treatment of edema and congestion in these patients. In view of the clinical courses included in the present study, we conclude that this disorder has a good prognosis. (Internal Medicine 38: 112-118, 1999) Key words: , , elder women,good prognosis

Introduction . The severity of mitral and tricuspid regur- gitation was globally assessed by dividing into three equal parts Idiopathic enlargement of the right was discussed by the distance from the valve orifice. The regurgitant jet was de- Bailey in 1955(1). This disorder may be an unusual congenital tected on color Doppler recording in the four-chamber view malformation. A review of the international literature disclosed and classified into one of the three regions (-: none, +: mild, that although several cases have been discussed since Bailey's ++:moderate, +++: severe). Definite pulmonary report (2-17), idiopathic bilateral atrial dilatation is not well was defined as more than 25 mmHg, which was calculated by known (18-20). We discuss eight patients with bilateral atrial Doppler echocardiography (-: none, +: mild). Patients with le- dilatation who presented with no other cardiac abnormalities sions known to be secondary to were ex- exceptfor atrial fibrillation. cluded. We investigated the clinical features and treatment course of each patient. Results of chest radiographs, electro- Methods cardiograms, and two-dimensional and color Doppler echocardiograms, when available, were examined. Four patients We studied the histories of eight patients, one man and seven had undergone magnetic resonance imaging. Wewerenot able women, treated for bilateral atrial dilatation within the past 13 to perform all of the tests on the more elderly patients. years at our hospital. The cardiothoracic ratio exceeded 65% Transesophageal echocardiography had been performed on in the patients studied. We used two-dimensional and color three patients. Cardiac catheterization and endomyocardial bi- Doppler echocardiographic findings to determine the follow- opsy from the right ventricle were performed on one patient. ing: 1) a left atrial short dimension of more than 55mm in the left parasternal window; 2) a right atrial short dimension of more than 55mm in the four-chamber view; 3) no disturbance Results in left ventricular wall motion and no structural abnormalities atient characteristics and clinical features (Table 1) of the tricuspid, mitral, aortic, and complexes; The patient group consisted of one manand seven women 4) no severe mitral regurgitation and/or aortic regurgitation, with a mean age of 70 years (range, 47 to 87 years). Dyspnea and no abnormal shunt flow confirmed by color Doppler wasP the chief complaint of four patients, and generalized edema

From the Division of , Department of Internal Medicine, Juntendo Urayasu Hospital, Juntendo University School of Medicine, Chiba Received for publication February 25, 1998; Accepted for publication November 9, 1998 eprint requests should be addressed to Dr. Mizuhiro Arima, the Division of Cardiology, Department of Internal Medicine, Juntendo Urayasu Hospital,

R Juntendo University School of Medicine, 2-1-1 Tomioka, Urayasu, Chiba 279-0021

112 InternalMedicineVol. 38, No. 2 (February 1999) Idiopathic Bilateral Atrial Dilatation

Table 1. Patient Characteristics and Clinical Features

C a s e N o . S e x A g e (y e a rs) c . c . C T R ( % ) E C G D ur a ti o n ( ye a rs ) C H F

1 F 8 0 D y sp n e a A F C R B B B L A D 3 7 + 2 F 4 7 E d e m a 6 5 A F I R B B B 1 6 3 F 8 7 E d e m a 7 0 A F L o w V o l t a g e 3 2 + 4 F 8 3 P alp itatio n 8 5 A F 4 0 D e a t h 4- 5 F 7 0 D y sp n e a 6 8 A F L V H 2 8 + 6 F 6 8 E d e m a 7 2 A F 3 8 + 7 F 7 4 D y sp n e a 7 8 A F I R B B B 3 4 8 M 5 2 D y sp n e a 6 8 A F l l

C. C : chief complaint, CTR: cardiothoracic ratio, ECG: electrocardiogram, Duration (year): duration after diag- nosis of cardiomegaly or arrhythmia, Case 4 died of cerebral infarction. CHF: congestive failure, -: none, +.CHF (Class III or Class IV on the NYHAscale), F: female, M: male, AF: atrial fibrillation, CRBBB:complete right , IRBBB: incomplete right bundle branch block, LAD: , Low Volt- age: low voltage in limb lead, LVH:left with inversion of and high voltage in left precordial lead.

was the chief complaint of three patients. Chest radiographs effective in these cases. One patient died of cerebral infarction showed markedly enlarged cardiac silhouettes from the time at the age of 83. Nopatient died as the result of congestive of the patients' first visit to our hospital. The range of the most . recent cardiothoracic ratios was from 65%to 88%, with a mean of 143%. The electrocardiograms showed atrial fibrillation in Changes in the cardiothoracic ratio (Fig. 1) all patients, incomplete right bundle branch block in two pa- Changes in the cardiothoracic ratio in the compensated state tients, complete right bundle branch block and left axis devia- of congestive heart failure are shown in Fig. 1. The cardio- tion in one patient, and left ventricular hypertrophy in one pa- thoracic ratio increased gradually. The range of cardiothoracic tient. Three patients were treated with digitalis because of atrial ratios from the first visit to our hospital was from 57%to 80%, fibrillation with rapid ventricular response. The meanduration with a meanof 68.5%, and the range from the most recent visit after diagnosis of cardiomegaly or arrhythmia was 29.5 years was from 65%to 88%, with a mean of74.3%. (range, 1 1 to 40 years). Five patients were categorized as Class III or Class IV on the NewYorkHeartAssociation scale (NYHA Findings of two-dimensional and color Doppler echocardio- scale) during their clinical course. Although six patients were grams (Table 2) admitted to our hospital because of worsening edemaor con- In each case, the echocardiogram showeda markedbilat- gestive heart failure, treatment with diuretics or digitalis was eral atrial dilatation with almost normal wall motion of both

CTR (%) 100_ -#- Case 1 -O- Case 2 90- ^^» -å²- Case3

-+^^å80- a----'* /V" ^^ ^ å -T- Case5

+ 70_ V V ^-^^f -V-Case6 i__ * ^g^PP^ ---Case7 V--~~~J*^^^ 60- CT -G- Case8

50-

40-

30-

'85 '86 '87 '88 '89 '90 '91 '92 '93 '94 '95 '96 '97 (Year)

Figure 1. Changes in the cardiothoracic ratio.

InternalMedicine Vol. 38, No. 2 (February 1999) 113 Arima et al

Table 2. Findings of Two-Dimensional and Color Doppler Echocardiograms

L V D d L V D s E F IV S L V P W L A D R A D T R M R P H Case No. (m m ) (m m ) (m m ) (m m ) (m m ) (m m )

1 4 8 3 0 0 .7 6 1 2 1 3 6 9 8 0 + + + + + 2 4 0 2 6 0 .7 2 1 0 l l 7 0 7 6 + + 3 4 9 3 2 0 .7 2 1 0 1 0 6 5 6 8 + 4 4 2 3 0 0 .6 4 l l 1 0 7 0 6 9 + + + + 5 4 2 2 8 0 .7 0 1 2 1 2 5 5 5 8 + + + 6 3 9 2 7 0 .6 6 l l l l 6 0 6 0 + + 7 5 0 3 0 0 .7 8 1 0 1 0 6 1 6 4 + + 8 4 1 2 5 0 .7 7 1 0 1 0 6 5 6 0 + +

LVDd: left ventricular diastolic dimension, LVDs: left ventricular systolic dimension, EF: ejection fraction, IVS: interventricular septal thickness, LVPW: left ventricular posterior wall thickness, LAD left atrial dimen- sion, RAD: right atrial dimension, TR: tricuspid regurgitation, MR: mitral regurgitation, PH: pulmonaly hyper- tension. ventricles. The left ventricular end diastolic dimension was gitation. Upon treatment with diuretics, the edema and con- 43.914.1 mm, and the ejection fraction was 0.72±0.05. Wall gestion disappeared, but the cardiomegaly improved only thickness of the left ventricle was within the normal limits in slightly. Follow-up chest radiographs are shown in Fig. 2. The all but one patient. The average dimension of the left atrium patient's cardiothoracic ratio increased gradually. Magnetic was 64.4±5.0 mm and that of the right atrium was 66.9±7.4 resonance imaging revealed a bilateral atrial dilatation in the mm. There was a tricuspid regurgitation signal in all of the transverse and coronal planes, but no other anatomic abnor- patients (severe in one, moderate in three, and mild in four), malities were observed (Fig. 3). Transesophageal echocardi- and a mild mitral regurgitation signal in three patients, as re- ography revealed a markedly enlarged bilateral atrium. There vealed on color Doppler echocardiography. No structural ab- were no structural abnormalities of the mitral and normalities of the tricuspid valve complex, such as tricuspid complex, such as valve prolapse or chordal rupture. Tricuspid valve prolapse or chordal rupture, were observed. It seems that and mitral regurgitation were due to the marked dilatation of tricuspid regurgitation was due to dilatation of the tricuspid the tricuspid and mitral annulus. The clinical picture was com- annulus. Although mild pulmonary hypertension of less than plicated by hypertension at the age of 72. Follow-up echocar- 40 mmHg was noted in five of the patients, no abnormal color diography showed the gradual progression of left ventricular shunt flow was observed in any of the patients. hypertrophy (interventricular septum 1 2 mm and posterior wall 13 mm) (Table 2). Casepresen tations .Case 1 No CaseNo.2 An 80-year-old woman had been diagnosed with cardiome- A 47-year-old woman had been hospitalized because of galy and arrhythmia at the age of 43, although she had not edema of the face and upper and lower extremities. Although complained of any symptoms. She first visited our hospital at she was diagnosed as having atrial fibrillation and cardiome- the age of 70 because of dyspnea on exertion at a Class II level galy during her second pregnancy at the age of 31, her first on the NYHA scale. Her blood pressure was 130/70 mmHg, pregnancy and delivery were normal. No heart murmurs or rales and she had an irregular pulse rate of 66 beats/min of atrial were audible. An electrocardiogram showed atrial fibrillation, fibrillation. A systolic murmur (Levin II/VI) was heard at the and chest radiographs showed cardiomegaly, with a car- left sternal border and the apex, but no rale was heard. She had diothoracic ratio of 66% on her first visit to our hospital. Trans- edema in the lower extremities, hepatomegaly, and jugular vein esophageal echocardiography demonstrated a greatly enlarged dilatation. An electrocardiogram showed atrial fibrillation with bilateral atrium, but both ventricles were normal. On color complete right bundle branch block and left axis deviation. Doppler echocardiography, a mild color signal for tricuspid Chest radiographs showed cardiomegaly with a cardiothoracic regurgitation was seen throughout the markedly enlarged right ratio of 82% and slight congestion in the bilateral fields. atrium. There was no abnormal shunt flow. Cardiac angiogra- Laboratory findings exhibited no abnormalities. The two-di- phy demonstrated a greatly enlarged bilateral atrium. Both ven- mensional and color Doppler echocardiograms revealed bilat- tricles contracted normally and were of normal size. The pul- eral atrial dilatation without left ventricle hypertrophy (inter- monary atrery was not dilated (Fig. 4). Cardiac catheterization ventricular septum 10mm and posterior wall 1 1mm), normal revealed no shunt. Pulmonary artery pressure was 16/8 mmHg, wall motion of both ventricles, and a mild color signal for mi- right atrial mean pressure was 4 mmHg, and right ventricular tral regurgitation and a severe color signal for tricuspid regur- pressure was 16/0 mmHg without dip and plateau. There was

114 Internal Medicine Vol. 38, No. 2 (February 1999) Idiopathic Bilateral Atrial Dilatation

Figure 2. Follow-up chest radiographs (Case 1). Chest radiographs taken in 1987 (upper left panel), 1992 (upper right panel), 1994 (lower left panel), and 1997 (lower right panel).

no evidence of Ebstein's anomaly,atrial or ventricular septal defect, or anomalous pulmonary venous return. Endomyo- cardial biopsy from the right ventricle revealed slight necrosis and degeneration of the myocardial cells. Interstitial fibrosis with slight infiltration of lymphocytes was seen (Fig. 5). Discussion

Idiopathic enlargement of the right atrium is a rare anomaly; only a handful of cases have been reported. Cardiomegaly is usually found incidentally on chest radiographs, and the af- fected heart shows idiopathic enlargement of the right atrium without any other cardiac abnormalities. A diagnosis of cardi- omegaly is often difficult to establish; postmortem findings occasionally confirm the clinical diagnosis. Idiopathic bilateral atrial dilatation is even more rare, with even fewer cases reported than with idiopathic enlargement of the right atrium. Unlike the criteria for the diagnosis of idio- pathic enlargement of the right atrium, the criteria for a diag- nosis of idiopathic bilateral atrial dilatation have not been es- Figure 3. Magnetic resonance imaging in the transverse plane tablished. Before the term "idiopathic" can be applied, all car- (Case 1). diovascular lesions known to produce bilateral atrial dilata- tion must be systematically excluded. Weselected eight cases Internal Medicine Vol. 38, No. 2 (February 1999) 115 Arima et al

Figure 4. Cardiac angiography (Case 2). Right atriography (upper left panel), right ventriculography (lower left panel), pulmonary arteriography (upper right panel), and levophase opacification of the left atrium and left ventricle (lower right panel). with the following characteristics: a cardiothoracic ratio of more à"dilatation (19, 20) and sudden death in idiopathic right atrial than 65%, bilateral atrial dilatation, and no bilateral ventricle enlargement have been described (2). Although five of our pa- dilatation on echocardiogram. Other causes of bilateral atrial tients had episodes of heart failure designated Class III or Class dilatation were excluded, including congenital or acquired val- IV on the NYHAscale, their clinical symptomsand signs dis- vular , shunt disease (, total anoma- appearedsoon after administration of diuretics and digitalis, lous pulmonary venous connection, etc.), and Ebstein's and the symptoms did not worsen in any of them. This demon- anomaly. Differential diagnosis from valvular disease is diffi- strates that effective medical treatment is available and that the cult. In the five reported cases of idiopathic bilateral atrial di- prognosis of this rare disorder is generally good. Keats and latation, a systolic murmur(maximumLevin II/VI) was au- Martt described an isolated case of dible in three cases. However, echocardiography revealed no associated with pregnancy (3). A review of the literature dis- abnormal findings in any of the four valves (19, 20). In four of closed five cases of idiopathic bilateral atrial dilatation seen our cases, a systolic murmur(less than Levin II/VI) was au- exclusively in women(19, 20). Seven of our eight cases were dible. Echocardiography and transesophageal echocardiography women.Therefore, atrial enlargement might be related to hor- revealed severe dilatation of both atriums. However, there were monal factors. In one reported case, a large free-floating throm- no abnormal findings in any of the four valves, nor in the cav- bus was found during surgery in the dilated right atrium (6). ity and wall motion of either ventricle. From these findings, Only one of our cases had a past history of embolization; this the systolic murmurseemed to be caused by tricuspid and mi- is due to the fact that patients were given sufficient antiplatelet tral regurgitation due to dilatation of the tricuspid and mitral treatment. annulus. It is still unknownwhether atrial fibrillation is the cause or Theclinical features of our cases are summarizedin Table the consequence of atrial dilatation (21). In one case, atrial 1. The duration after diagnosis of cardiomegaly or arrhythmia enlargement could have occurred as a consequence of atrial was lengthy. Congestive heart failure in idiopathic bilateral atrial fibrillation (22). In another case, the enlarged left atrium was

116 Internal Medicine Vol. 38, No. 2 (February 1999) Idiopathic Bilateral Atrial Dilatation

Figure 5. Endomyocardial biopsy from the right ventricle (Case 2). Histological section stained with hema- toxylin and eosin (x 200) is shownin the left panel, which revealed slight necrosis and degeneration of the myocar- dial cells with infiltration of lymphocytes. Histological section stained with azan (x 100) (right panel), revealed interstitial flbrosis.

likely to have been the cause rather than the consequence of tion was not performed in the other patients involved in our atrial fibrillation (23). In a third reported case, left atrial dilata- study. tion mayhave represented both the cause and the consequence Most authors have considered idiopathic enlargement of the of atrial fibrillation (24). A review of electrocardiogram find- right atrium to be congenital, because it is usually diagnosed ings showedthat atrial fibrillation was present in all the pa- in early childhood. In the present study, in addition to congeni- tients we studied at their first visit to our hospital. It wasuncer- tal and ventricular diastolic dysfunction, we also suggest that tain when the atrial dilatation began. Wedo not knowwhich long-standingatrial fibrillation contributes to the development abnormality occurred first, cardiomegaly or atrial fibrillation. of bilateral atrial dilatation. Histologically, idiopathic enlarge- However, according to successive chest radiographs, the ment of the right atrium revealed a partial loss of atrial muscu- lar fibers, irregular thickening of muscle fibers, and lipoma- Thissuggeststhatcardiothoracicbilateralatrial ratio gradually increaseddilatation in wasthe topresentsome cases.extent tous degeneration and lymphocytic infiltration of the atrial wall influenced by long-term atrial fibrillation. Right atrium enlarge- (5-8). These findings demonstrate that the pathogenesis of id- ment due to both long-term atrial fibrillation and, presumably, iopathic enlargement of the right atrium is not only congenital, right ventricular diastolic dysfunction caused by aging, could but also that changes may occur after . The etiol- dilate the tricuspid valve annulus and cause tricuspid regurgi- ogy of idiopathic bilateral atrial dilatation is unclear, but the tation (4). The cardiac catheterization that was performed in dilatation does not usually develop until gross cardiomegaly one patient revealed bilateral normal ventricular end-diastolic manifests on chest radiographs unless an underlying cardiac pressure. Therefore, this particular case did not support the disease is present. One of the patients in our study underwent presence of bilateral ventricular diastolic dysfunction. Wewere endomyocardialbiopsies from the right ventricular wall. These able to exclude the restrictive (endomyo- biopsies showed necrosis and degeneration of the myocardial cardial fibrosis, , Fabry's disease, etc.), which in- cells, and interstitial fibrosis with infiltration of lymphocytes. volve abnormal ventricular diastolic function. However, we These histological findings suggest cardiac injury, including cannot categorically deny the presence of slight ventricular myocarditis.One study reported that idiopathic enlargement diastolic dysfunction caused by aging, as cardiac catheteriza- of the right atrium was associated with moderate dilatation of

InternalMedicine Vol. 38, No. 2 (February 1999) 117 Arima et al the left atrium; in histological findings, infiltration of lympho- 58: 683-688, 1994. 5) Marin-Garcia J. Idiopathic dilation of the right atrium. Chest 79: 378- cytes was found in the right atrium and, to a lesser degree, in 379, 1981. the left atrium and in both ventricles as well (9). Since we could 6) Sheldon WC, Johnson CD, Favaloro RG. Idiopathic enlargement of the not perform endomyocardial biopsy of the atrium, but instead right atrium: report of four cases. AmJ Cardiol 23: 278-284, 1969. biopsied the right ventricle, it remains unclear whether the in- 7) Pastor BH, Forte AL. Idiopathic enlargement of the right atrium. AmJ flammatory change in the right ventricle might have been re- Cardiol 8: 513-518, 1961. lated to atrial dilatation. It is very difficult to ascertain whether 8) Saigusa M, Morimoto K, Koike T, Hori T, Sato T. Idiopathic enlargement of the right atrium. Jpn Heart J 3: 373-379, 1962. inflammatory changes produced the atrial dilatation observed 9) Terada T, Oiwake H, NakanumaY, Ohta G, Nishino T. An case of n our patients. idiopathic enlargement of the right atrium, and a review of the literature. In conclusion, since some cases of idiopathic enlargement Acta Pathol Jpn 38: 361-370, 1988. of the right atrium were associated with dilatation of the left 10) Sumner RG, Phillips JH, Jacoby WJ Jr, Tucker DH. Idiopathic enlarge- atrium, idiopathic enlargement of the right atrium was not ab- ment of the right atrium. Circulation 32: 985-991, 1965. 1 1) Pernot C, Hoeffel JC, Jacob F. Idiopathic dilatation of the right atrium solutely inconsistent with bilateral atrial dilatation. Both idio- revealed in childhood by dysrhythmias. Pediatr Radiol 6: 52-54, 1977. pathic enlargement of the right atrium and idiopathic bilateral 12) Beder SD, Nihill MR, McNamara DG. Idiopathic dilation of the right atrial dilatation are extremely rare. To our knowledge, the atrium in a child. Am Heart J 103: 134-137, 1982. 13) Maione S, GiuntaA, Betocchi S, Ferro G, Vigorito C, Chiariello M. Two- present study is the first to describe eight cases of idiopathic dimensional echocardiography in idiopathic enlargement of the right bilateral atrial dilatation. By histological investigation, inflam- atrium. Reliability and limitations. Cardiology 70: 216-222, 1983. matory change was found in the right ventricle in one case, but 14) Marin-Garcia J, Allen RG. Idiopathic dilatation of the right atrium: post- the etiology of idiopathic bilateral atrial dilatation could not be operative follow-up in a child. J Pediatr Surg 18: 196-198, 1983. distinctly clarified. Our study of idiopathic bilateral atrial dila- 15) Bekassy SM, Konya L. Idiopathic dilatation of the right atrium. A case tation presents a variegated clinical picture. The clinical fea- report. Scand J Thorac Cardiovasc Surg 18: 191-192, 1984. tures of this disorder suggest that most cases occur in women, 1 6) Iwase M, Hurui H, Miyaguchi K, et al. Two-dimensional echocardiography therefore, atrial enlargement might be related to hormonal fac- and magnetic resonance imaging in diagnosis of idiopathic dilation of the right atrium. Am Heart J 120: 1231-1233, 1990. tors. Over the long clinical history of the patients after the di- 17) TeradaY, Mitsui T, Yamamoto T. Enlargement of the right atrium associ- agnosis of cardiomegaly or arrhythmia, diuretics or digitalis ated with dermatomyositis. Ann Thorac Surg 59: 263-264, 1995. offered good results in the treatment of edema and congestion. 18) Rogers WR, Wittels B. Extreme bilateral atriomegaly. Review of the lit- We conclude that this disorder has a good prognosis. erature and report of a case. Circulation 15: 434-441, 1957. i 19) Rath S, Schneewiess A, Battler A, Har-Zahav Y, Neufeld HN. Idiopathic s evere bilateral atrial dilation. Am J Cardiol 52: 426-427, 1983. References 20) Maeda S, Katoh Y, Ohkawa S, Takubo K, Esaki Y. Marked bilateral atrial d ilation of unknown cause. J Cardiol 26: 33-38, 1995. 21) AboafAP, Wolf PS. Paroxysmal atrial fibrillation. A common but ne- 1) Bailey CP. Surgery of the Heart. Lea and Febiger Publisher, Philadelphia, 1955,p. 413. g 22) Sanfilippolected entity.AJ, ArchAbascalInternVM,MedSheehan156: 362-367,M, et al. 1996.Atrial enlargement as a 2) Tenckhoff L, Stamm SJ, Beckwith JB. Sudden death in idiopathic (con- genital) right atrial enlargement: postmortem findings and review of cases. consequence of atrial fibrillation: a prospective echocardiographic study. C Circulation 40: 227-235, 1969. 23) PsatyirculationBM, Manolio82: 792-797,TA, Kuller1990. LH, et al. Incidence of and risk factors for 3) Keats TE, Martt JM. Selective dilatation of the right atrium in pregnancy. a AmJ Roentgenol 91: 307-310, 1964. 24) Andersentrial fibrillationJS, Egebladin older H,adults.AbildgaardCirculationU, Aldershvile96: 2455-2461,J, Godtfredsen1997. J. 4) Iga K, Konishi T, MatsumuraT, Miyamoto T, Kijima K, Gen H. Mark- edly enlarged right atrium associtated with physical signs of tricuspid Atrial fibrillation and left atrial enlargement: cause or effect? J Intern regurgitation: a cause of congestive heart failure in the elderly. Jpn Circ J Med 229: 253-256, 1991.

118 Internal Medicine Vol. 38, No. 2 (February 1999)